People living with CF experience a slow decline in lung function and multi-organ complications may occur over a long period. In the prolonged chronic phase of the illness, patients undergo intensive regimes of treatment and predicting prognosis is difficult. Lung transplant is not an option for all individuals with CF. Variable disease progression can mean that the timing of death may be difficult to predict.
Providing optimal end-of-life care may be challenging due to the variation in disease, dyspnoea, pain, congestion and anxiety being the most prevalent symptoms. Ideally, this would be provided by a specialised palliative care team. In CF, active and palliative care usually occurs simultaneously, and in some cases, life-sustaining treatment will occur while awaiting lung transplant.
Advance Care Planning is particularly important for people living with CF because of the unique aspects of the disease. Planning should reflect and respect the individual’s decisions as lung disease progresses.
Health Professional Resources:
- Advance Care Planning for Health Care Workers (Advance Care Planning Australia)
- Advance Care Planning in Western Australia (Advance Care Planning Australia)
- Palliative Care Information Booklet (Palliative Care WA)
- Advance Care Planning: A Patient’s Guide (WA Department of Health)